Deck 2 Flashcards
(26 cards)
Q. What occurs in vasculitis?
A. Inflammation and necrosis of blood vessel walls with subsequent impaired blood flow
a. Vessel wall destruction: Perforation + haemorrhage into tissues
b. Endothelial injury: Thrombosis + ischaemia/infarction of dependent tissues
Q. What cells are seen in histological slides of vasculitis?
A. Vessel wall infiltration: neutrophils, mononuclear cells, giant cells
B. Dissolution of leucocytes
How does vasculitis present?
A. No single typical presentation: Systemically unwell, fever, arthralgia/arthritis,
rash, weight loss, headache, footdrop, major event eg stroke, bowel infarction
B. Easily confused with other diseases-mimickers: Must be excluded to ensure
correct treatment -
Sepsis-SBE, hepatitis
Malignancy
Other-eg cholesterol emboli
Q. In vasculitis which sized blood vessels are associated with Anti-neutrophil
cytoplasmic antibodies? (ANCA) What are they? What are the two major patterns?
A. Small/medium vessel vasculitis
B. Specific antibodies for antigens in cytoplasmic granules of neutrophils and
monocyte lysosomes
C. Detected with indirect immunofluorescence
D. Two major patterns: Cytoplasmic ANCA (c-ANCA) and Peri-nuclear ANCA (p-
ANCA)
Q. Name two primary large vessel vasculitis conditions, describe the classical
presentation
A. Giant cell (temporal arteritis: granulomatous arteritis of aorta and larger vessels- extracranial branches of carotid arteries, affects ophthalmic artery
a. Incidence: > 50 yrs, increases with age, more common in women
b. Temporal artery tenderness/decreased pulsation
c. Presents: headache, scalp tenderness, jaw claudication, acute blindness- medical emergency, non-specific malaise, associated symptoms of polymyalgia rheumatic, risk of CVA
d. Diagnosis: temporal artery biopsy
e. ANCA negative
B. Takyasu’s arteritis
Q. Why may patients with temporal arteritis (giant cell arteritis) present to
ophthalmology?
A. Sudden, painless, monocular and severe vision loss. May be preceded by
transient visual loss
B. Optic disc becomes pain and swollen, often with flame-shaped haemorrhages at
the margin
C. Curtain loss of vision
D. Due to granulomatous thickening of the inner portions of the branches of the
external carotid arteries
Q. How should temporal arteritis (giant cell arteritis) be treated?
A. Prompt corticosteroids, dramatic response usually seen in 48hrs
B. Immunosuppression
Q. Name two primary large vessel vasculitis conditions, describe the classical presentation
A. Granulomatosis with polyangiitis (GPA), (Wegener’s Granulomatosis)
a. Necrotizing, granulomatous vasculitis of arterioles, capilleries and post capillary venules
b. Associated with c-ANCA
c. 25-60 years old
d. Affects vasculature of (all organ systems) – resp, kidney, skin, NS, eye
e. Eye disease, saddle nose deformity
B. Churg Strauss
C. Microscopic polyangiitis
Q. Name 3 organ systems GPA may affect and the typical manifestation
A. Upper respiratory tract: sinusitis/otitis/nasal crusting bleeding
B. Lungs: pulmonary nodules/haemorrhage
C. Kidney: Glomerulonephritis, (haematuria/proteinura)
D. Skin: purpura/ulcers
E. Nervous system: mononeuritis multiplex/CNS vasculitis
F. Eye: proptosis/scleritis/episcleritis/uveitis
G. Other: synovitis/pericarditis/
Q. What occurs in Granulomatosis with polyangiitis (GPA)-eye disease?
A. Marked bilateral periorbital edema
B. Chemosis (swelling of conjuctiva) of the left eye secondary to local
granulomatous inflammation
C. Granulomatous orbital disease
Q. Who is at an increased risk of crystal disease/infection/gout?
A. Pts who are: diabetic, obese, hypertensive, alcohol
B. Infection: bacteraemia, age, immunosuppressed, (aspirate joint: crystal shape – can aid diagnosis)
Q. Name 3 differences between synovial, fibrous and cartilaginous joints, give an example of each
A. Synovial: 2 articulating bone surfaces –highly mobile - hyaline cartilage, fibrous capsule lined with synovium, joint space filled with synovial fluid – knee (bicondylar), ankle (hinge-type), hip (ball and socket)
B. Fibrous: connected by dense connective tissue (mostly collagen), fixed joint, (sutures-skull), between tibia and fibula, teeth to jaw bones
C. Cartilaginous: connected by cartilage (more movement than fibrous, less than synovial) e.g. manubriosternal joint, intervertebral discs and pubic symphysis
Q. What occurs in rheumatoid arthritis?
A. Inflammation – chronic inflammatory reaction, lymphocytes/macrophages/plasma cells
B. Proliferation: tumour like mass (pannus), grows over articular cartilage
Q. Describe four features of Rh arthritis presentation
A. Symmetrical arthropathy
B. Hands and feet > 80% cases
C. Early morning stiffness
D. Progressive inflammation of joint
E. Pain, loss of function, deformity and damage
F. Extra-articular involvement: lungs, heart, GI, skin, eyes, kidneys
G. Symptoms: joint pain worse in morning (improvement with use), morning
stiffness (several hours), loss function, general fatigue
H. Nodules, bursitis, tenosynovitis, muscle wasting
I. Palpable lymph nodes, spleen, anaemia
Q. Describe possible manifestations of Rh arthritis of the eyes
A. Sicca (dry eyes), secondary siorgren’s syndrome, episcleritis, scleritis
Q. Describe 3 possible neurological manifestations of Rh arthritis
Mild primarily sensory peripheral neuropathy (legs>arms)
B. Entrapment neuropathies: soft tissue swelling, carpel tunnel, elbow, popliteal
space, tarsal tunnel
C. Cervical instability/myelopathy/atlanto-axial subluxation
Q. Describe possible manifestations of Rh arthritis of the lung
Pleural effusion, diffuse fibrosing alveolitis, rheumatoid nodules, caplan’s syndrome (rare –coal exposure), small airways disease
Q. Describe some possible manifestations of Rh arthritis of the heart
Pericardial rub, pericarditis, pericardial effusion
Describe some possible manifestations of Rh arthritis of the kidney
Amyloidosis, analgesic nephropathy
Q. Describe some possible manifestations of Rh arthritis of the skin
Vasculitis, small digital infarcts along nailbeds, abrupt onset of ischaemic mononeuropathy or progressive scleritis typical of rheumatoid vasculitis, Seropositive usually, persistently active disease (can occur when joints inactive)
Q. What investigations – rheumatoid arthritis
A. Anaemia
B. High ESR/CRP (related to inflammation)
C. Positive RF 80% (Rheumatoid factor- antibody)
D. Anti-CCP 80%
E. ANA <50%
F. Negative for all – 20% of cases
- Q. Name 3 connective tissue diseases
A. Inherited: Marfan’s, Ehler Danlos syndrome
B. Auto-immune (inflammatory): systemic lupus erythematosus (SLE), systemic sclerosis (scleroderma), polymyositis and dermatomyositis, ‘overlap’ syndromes and undifferentiated autoimmune rheumatic disease
Q. Who is at a greater risk of SLE?
A. 90% women (?oestrogen), afro-Caribbean’s, genetic association (some drugs, UV,
EBV?)
B. Varied clinical manifestations – symmetrical
3 SLE symptoms
Varied clinical manifestations – symmetrical small-joint arthralgia (Pain) and skin
manifestations are common: … fever, malaise, depression (nonspecific), Discoid
rash, photosensitive rash, alopecia, mouth ulcers, fatigue, arthritis, mucosal
ulceration, depression
C. Neuro: headaches, aseptic meningitis, polyneuropathy, psychosis
D. Haematological: anaemia, thrombocytopenia, neutropenia, lymphopenia
